A Case of Tuberous Sclerosis Complicated with Massive Bleeding of Renal Angiomyolipoma

Korean Journal of Nephrology 2007;26(4):485-490.

Sun Jeong Byun, M.D.1, Kyu-Beck Lee, M.D.1 , Yong Su Lee, M.D.1 , Hang Kim, M.D.1, Hyun Pyo Hong, M.D.2, Seung Kwon Kim, M.D.2, Young Rae Lee, M.D.2, Sung Wan Chae, M.D.3 and Jin Hee Son, M.D.3

Department of Internal Medicine¹
Department of Diagnostic Radiology2; Department of Pathology³
College of Medicine, Kangbuk Samsung Hospital Sungkyunkwan University School of Medicine, Seoul, Korea

신장 혈관근지방종의 대량출혈을 일으킨 결절성 경화증 1예

변선정1

성균관대학교 의과대학 강북삼성병원 신장내과1, 방사선과2 , 해부병리과3

Abstract

Tuberous sclerosis (TSC) is a systemic, autosomal dominant disorder resulting from mutations in one of two genes, TSC1 (encoding hamartin) or TSC2 (enconding tuberin). TSC causes seizure, mental retardation and hamartomatous tumors in multiple organs, including facial angiofibromas, cortical tubers, pulmonary lymphangiomatosis, renal angiomyolipomas and polycystic kidney disease. Renal angiomyofibromas may cause serious complications such as life threatening retroperitoneal hemorrhage or hematuria. The following is a report concerning a 41-year-old man with TSC who suffered spontaneous hemorrhage within the angiomyofibroma of the left kidney and underwent curative selective renal embolization. Then larger angiomyolipoma was suggested to be more likely to bleed, so secondary prophylactic selective renal embolization was done into five angiomyolipomas of the right kidney. After selective embolization, tumor size decreased and renal function was preserved. This patient did not show neurologic abnormality and family history of tuberous sclerosis. However, the brain magnetic resonance imaging revealed typical signs of tuberous sclerosis, and the computerized tomography of the abdomen showed bilateral renal angiomyolipomas and polycystic renal lesion. Herein we present a rare case of bilateral renal angiomyolipomas with spontaneous hemorrhage and preserved renal function after curative and prophylactic selective embolization.

Key Words: Tuberous sclerosis, Angiomyolipoma, Polycystic kidney disease